限制型心肌病患者心内膜心肌活检的组织学病理特点及心功能变化

目的探讨限制型心肌病患者心内膜心肌活检的组织学病理特点以及相关心功能变化。方法对我院急诊科2010年1月至2013年10月收治的40例限制型心肌病患者（心肌病组）进行心内膜心肌活检,并做实验室检查、右心导管以及心肌病理检查,对其结果进行分析,并与40例正常对照组比较。结果心肌病组的B型利钠肽（BNP）、肌酸激酶同工酶（CK-MB）和肌钙蛋白I（TnI）明显高于对照组（P均〈0.05）;心肌病组的右心房压、右心室收缩压、右心室舒张末压和肺动脉楔压明显高于对照组右心房压：（18.7±4.5）mmHg vs（10.2±1.4）mmHg;右心室收缩压：（46.5±5.6）mmHg vs（34.7±3.7）mmHg;右心室舒张末压：（16.3±4.6）mmHg vs（8.6±2.6）mmHg;肺动脉楔压：（28.6±5.3）mmHg vs（14.2±3.8）mmHg,P均〈0.05];20例诊断为心肌淀粉样变性,6例诊断为嗜酸性粒细胞增多综合征累及心肌,14例心内膜心肌活检提示存在心肌病变。结论淀粉样变性、嗜酸性粒细胞浸润和特发性心肌病是限制型心肌病的主要病因。心内膜心肌活检对于限制型心肌病的病因诊断意义重大。

Histological and pathological features,cardiac function of restrictive cardiomyopathy by endomyocardial biopsy

Objective To explore the histological and pathological features of restrictive cardiomyopathy by endomyocardial biopsy, as well as cardiac function changes. Methods From January 2010 to October 2013, 40 patients of restrictive cardiomyopathy from the Emergency Department（cardiomyopathy group） were treated by endomyocardial biopsy, laboratory tests, right heart catheterization and cardiac pathology. The results were analyzed and compared with that of 40 healthy individuals（the control group）. Results The levels of B-type natriuretic peptide（BNP）, CK-MB and TnI of cardiomyopathy group were significantly higher than those of the control group（P〈0.05）;The right atrial pressure, right ventricular systolic pressure, right ventricular end-diastolic pressure and pulmonary artery wedge pressure of cardiomyopathy group were significantly higher than the control group： right atrial pressure：（18.7±4.5） mmHg vs（10.2±1.4） mmHg; right ventricular systolic pressure：（46.5±5.6） mmHg vs（34.7±3.7） mmHg;right ventricular end-diastolic pressure（16.3±4.6） mmHg vs（8.6±2.6） mmHg; pulmonary artery wedge pressure（28.6±5.3） mmHg vs（14.2±3.8） mmHg, all with P〈0.05. Twenty patients were diagnosed as cardiac amyloidosis, 6 were diagnosed as eosinophilic increased syndrome involving myocardium, and 14 suggested the presence of myocardium disease. Conclusion Amyloidosis, eosinophil infiltration and idiopathic cardiomyopathy are major causes of restrictive cardiomyopathy. Endomyocardial biopsy has great significance for the diagnosis of restrictive cardiomyopathy